A 21-year-old female was found to have bilateral multiple irregularly branching interconnected subretinal thread-like black lines radiating from the optic disc [Figure 1]a and [Figure 1]b. Visual acuity was 20/20 in either eye. The fundus fluorescein angiogram (FFA) images are given in [Figure 1]c and [Figure 1]d.

Figure 1: (a and b) Fundus photographs of the right eye and the left eye. (c and d) Corresponding fundus fluorescein angiograms

[Figure 1] shows angioid streaks around the optic disc and typical peau d'orange appearance temporal to the fovea. FFA reveals window defects corresponding to the angioid streaks. The fovea is healthy, and there was no evidence of choroidal neovascular membrane (CNVM)

Pseudoxanthoma elasticum (PXE), Ehlers Danlos syndrome, Paget's disease, sickle cell anemia, acromegaly, diabetes mellitus, and other systemic diseases should be ruled out in patients with angioid streaks.[1] This female had asymptomatic coalescing yellowish plaques on the posterolateral side of her neck [“plucked chicken” appearance, [Figure 2]. Biopsy followed by hematoxylin-eosin and Verhoeff Van Gieson staining showed irregularly clumped calcified elastic fibers in reticular dermis confirming PXE. The patient was explained to follow-up with the ophthalmologist regularly and to report urgently in case of metamorphopsia or vision loss which may denote the development of a CNVM.

Discussion

PXE is a genetic disorder involving multiple systems.[2] Histopathologically, it is characterized by the collection of calcified, degenerated, and fragmented elastic fibers in the Bruch's membrane, elastic media and intima of arteries, endocardium, and the dermis. Visual loss is generally due to the development of a secondary CNVM. Other retinal features include comet lesions, chorioretinal atrophy, pattern dystrophy-like changes, debris collection under the retinal pigment epithelium; reticular pseudodrusen; and subretinal fluid independent of CNVM.[3],[4] The plucked chicken (Moroccan-leather) appearance of the skin is most commonly seen at the neck, the axilla, and antecubital fossa. Involvement of the fragile arteries and the cardiac valves results in intermittent claudication, renal artery stenosis, gastrointestinal hemorrhage, hemorrhage from the urinary tract and brain; and mitral valve prolapse.

Conclusion

PXE is an important association of angioid streaks and should be ruled out in all such cases as it has various serious systemic implications.

Acknowledgments

The authors would like to thank Trina Sengupta Tripathy for her immense support for preparing the manuscript.